Porta hepatic schwannoma: case report and a 30-year review of the literature yielding 15 cases
Yin et al. World Journal of Surgical Oncology
Porta hepatic schwannoma: case report and a 30-year review of the literature yielding 15 cases
Sheng-yong Yin 0 1 2 3
Zheng-long Zhai 0 1 2 3
Kui-wu Ren 1 2 3
Yun-chuan Yang 1 2 3
Da-long Wan 1 2 3
Xiao-yan Liu 4
Li-jun Wang 4
Shu-sen Zheng 1 2 3
0 Equal contributors
1 Key Laboratory of Combined Multi-organ Transplantation, Ministry of Public Health, Key Laboratory of Organ Transplantation, Division of Hepatobiliary and Pancreatic Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University , Hangzhou 310003Zhejiang Province , China
2 Department of Hepatobiliary and Pancreatic Surgery, First Affiliated Hospital, Zhejiang University School of Medicine , 310003 Hangzhou , China
3 Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases, First Affiliated Hospital, Zhejiang University School of Medicine , 310003 Hangzhou , China
4 Department of Pathology, First Affiliated Hospital, Zhejiang University School of Medicine , 310003 Hangzhou , China
Background: Schwannomas located in the periportal region are extremely rare. Only 14 cases have been reported in the medical literature worldwide. Cases of porta hepatic schwannomas reported in the literature worldwide were reviewed. As a result, it is very challenging for surgeons to make a preoperative diagnosis due to its rarity and nonspecific imaging manifestations. Case Presentation: A 57-year-old Chinese female was admitted to our institution with complaint of upper abdominal distension and the abdominal CT in the local hospital revealed a hypodense mass in the porta hepatis. A fine needle aspiration (FNA) was made to confirm the diagnosis, but the result was just suggestive of spindle cell neoplasia. Eventually, the patient underwent surgery and postoperative pathology confirmed schwannoma in porta hepatis. The patient recovered uneventfully with no evidence of recurrence after a follow-up period of 41 months. Conclusions: It is essential for the final diagnosis of porta hepatic schwannomas to combine histological examination with immunohistochemistry after surgery. The main treatment of porta hepatic schwannomas is complete excision with free margins and no lymph node dissection. In some cases, biliary reconstruction or the proper hepatic and the gastroduodenal artery resection was performed because the tumor was inseparably attached to the extrahepatic bile duct or the proper hepatic and the gastroduodenal artery. Malignant transformation of schwannomas is very rare and the overall prognosis is satisfactory.
Schwannomas; Hepatoduodenal ligament; Porta hepatis; Proper hepatic artery; S-100
Background
The periportal region is a complex anatomic region
between the superior aspect of the first portion of the
duodenum and the porta hepatis, including the
hepatoduodenal ligament and the extrahepatic bile duct,
portal vein, hepatic artery, autonomic nerve fibers, and
lymph nodes [
1
]. When patients exhibit symptoms of
abdominal pain or jaundice or show no symptoms, and
a mass in the periportal region is detected in image
scans, the diagnosis of a cholangiocarcinoma or a
gastrointestinal stromal tumor (GIST) may be considered first.
However, in rare cases, the diagnosis is of a porta
hepatic schwannoma. Porta hepatic schwannomas are
extremely rare, with only 14 cases reported in the
literature to date. Of the 14 cases, the mean age of the
patients was 45 years (range 29–74 years), and the
malefemale ratio was 6:8. The literature depicted tumors
varying from 2.2 cm (the average tumor diameter line)
to 7.5 cm in size, with a median of approximately
4.8 cm. For the mass in porta hepatic, it is difficult to
perform a biopsy because of vessel interposition. So, the
surgeons are inclined to make a fine needle aspiration
(FNA) because of lower risk of bleeding. In the 14 cases,
the FNA was operated only for two patients, and none
underwent biopsy before surgery. But, the FNA usually
fails to provide the immunohistochemistry, which is
imperative to make a diagnosis for the mesenchymal
tumors. In most cases, the final diagnosis of schwannoma
was confirmed by postoperative pathological examination.
Herein, we report a case of schwannoma located in
the porta hepatis and review cases reported in the
literature worldwide for a better understanding of the clinical
and pathological features of porta hepatic schwannomas.
Case presentation
On October 12, 2011, a 57-year-old Chinese female was
admitted to our institution with complaint of upper
abdominal distension for 2 weeks. She denied any
abdominal trauma or surgeries in her life. No specific
findings were noted in her family history and personal
history. And, she was taking no medications. Before this
treatment, she went to the local hospital where an
abdominal ultrasound was performed revealing a mass in
the head of the pancreas. No abnormalities were
detected upon clinical examination and in laboratory
investigations. The computer (...truncated)